Project description:We present a case of common carotid artery (CCA) diaphragm. To our knowledge, this is the first reported case in the imaging literature.A 33-year-old woman presented herself to an annual health examination without any report of ill health.A stenosis with aneurysm dilation on the proximal part of the right CCA was revealed on ultrasound and CTA, by which a diaphragm-like structure on the stenosis lesion was clearly identified. An anatomic variant of artery was first considered.The patient was followed up closely.There was no progression of her CCA stonosis 1 year later.This unusual and previously unreported case is presented to alert vascular sonographers and radiologists to its existence.
Project description:Bullous pemphigoid (BP) is a rare, life-threatening autoimmune blistering disease with pruritus and tension blisters/bullous as the main clinical manifestations. Glucocorticosteroids are the main therapeutic agents for it, but their efficacy is poor in some patients. Tofacitinib, a small molecule agent that inhibits JAK1/3, has shown incredible efficacy in a wide range of autoimmune diseases and maybe a new valuable treatment option for refractory BP. To report a case of refractory BP successfully treated with tofacitinib, then explore the underlying mechanism behind the treatment, and finally review similarities to other cases reported in the literature. Case report and literature review of published cases of successful BP treatment with JAK inhibitors. The case report describes a 73-year-old male with refractory BP that was successfully managed with the combination therapy of tofacitinib and low-dose glucocorticoids for 28 weeks. Immunohistochemistry and RNA sequencing were performed to analyze the underlying mechanism of tofacitinib therapy. A systematic literature search was conducted to identify other cases of treatment with JAK inhibitors. Throughout the 28-week treatment period, the patient experienced clinical, autoantibody and histologic resolution. Immunohistochemical analysis showed tofacitinib significantly decreased the pSTAT3 and pSTAT6 levels in the skin lesions of this patient. RNA sequencing and immunohistochemical testing of lesion samples from other BP patients identified activation of the JAK-STAT signaling pathway. Literature review revealed 17 previously reported cases of BP treated with four kinds of JAK inhibitors successfully, including tofacitinib (10), baricitinib (1), upadacitinib (3) and abrocitinib (3). Our findings support the potential of tofacitinib as a safe and effective treatment option for BP. Larger studies are underway to better understand this efficacy and safety.
Project description:BackgroundRight coronary artery (RCA) injury is a rare complication of valvular surgery. However, complications should be considered, due to the significant clinical consequences. Identifying the coronary injury type and understanding the underlying pathophysiological mechanisms is essential to managing these complications.Case summaryThe case of a 59-year-old man who underwent conservative mitral valve surgery with tricuspid valve annuloplasty is presented. The early post-operative period was complicated by acute coronary syndrome with inferior persistent ST-segment elevation. A coronary angiogram confirmed critical RCA hazy lesions, raising the suspicion of coronary kinking. To confirm the underlying mechanism for these lesions and determine the best treatment strategy, endocoronary imaging was performed, revealing coronary kinking of the RCA. Based on the persistent acute ischaemia, a long-lasting drug-eluting stent (DES) was implanted in the lower and upper knees of the RCA. After angioplasty, electrocardiography showed regression of the ST-segment elevation. Ten days later, coronary angiography and optical coherence tomography showed good results. The patient recovered from his myocardial infarction.DiscussionOnly a few reports describe the use of endocoronary imaging for diagnosing coronary artery injury after tricuspid annuloplasty. The variety of lesion types that could underlie a single post-operative myocardial infarction makes endocoronary imaging a relevant technique to guide management strategy and optimize DES implantation.
Project description:BackgroundAgenesis of the internal carotid artery (ICA) is a rare congenital abnormality, sporadically reported to be associated with a combined congenital hypopituitarism. Nevertheless, only a few cases have been extensively described, and none of these have been characterized by an isolated growth hormone (GH) deficiency.Case presentationHere, we describe a 17-year old boy referred to our hospital for fatigue, decreased muscle strength and severe headache reported after the cessation of rhGH treatment for a GH deficiency diagnosed at the age of 2 years and 3 months. Magnetic resonance imaging (MRI) showed an adenohypophyseal hypoplasia with a lack of posterior pituitary hyperintensity, whereas MRI angiography indicated the absence of a normal flow void in the left ICA. Endocrinological tests confirmed the GH deficiency (GH peak after growth-hormone-releasing hormone (GHRH) + arginine: 2.42 ng/mL) with a very low IGF-I value (31 ng/mL) and normal function of other pituitary axes.ConclusionTo the best of our knowledge this is the first confirmed case of an isolated GH deficiency in a patient with ICA agenesis. The presence of an isolated pituitary deficit is unlike to be considered only as an effect of hemodynamic mechanism, suggesting a role for genetic factor(s) as a common cause of these two rare birth defects. Further studies could clarify this issue and the underlying mechanisms to better understand the etiopathogenetic characteristics of this disorder.
Project description:Traumatic pseudoaneurysms are uncommon vascular lesions that can result from traumatic injuries to the nasal and facial areas. They pose a significant clinical challenge due to their potential to cause recurrent and life-threatening epistaxis. Understanding the underlying vascular anatomy, causes, and treatment options is essential for optimizing patient outcomes. In this case report, we present a 55-year-old man who developed a traumatic pseudoaneurysm of the sphenoid sinus, arising from the cavernous segment of the internal carotid artery following a traumatic incident. This case was successfully managed with stent-assisted coil embolization.
Project description:BackgroundAlthough it is well known that internal carotid-posterior communicating artery (ICA-PcomA) aneurysms compress the oculomotor nerve and cause nerve palsy, cases of ICA-PcomA aneurysms splitting the oculomotor nerve are extremely rare.Case descriptionWe present the rare case of an asymptomatic, growing, left-sided ICA-PcomA aneurysm that was confirmed to split the oculomotor nerve. We report the clinical course and discuss the underlying mechanism. The oculomotor nerve, which is an aggregate of multiple fibers, exhibits age-related loss of compactness in the arrangement of its nerve fibers.ConclusionWe speculate that injury to the nerve fibers by aneurysmal compression was avoided because of the rare phenomenon of splitting of the oculomotor nerve.
Project description:Members of a disintegrin and metalloproteinases with thrombospondin motif (ADAMTS) family have been implicated in various vascular diseases. However, their functional roles in early embryonic vascular development are unknown. In this study, we showed that Adamts18 is highly expressed at E11.5-E14.5 in cells surrounding the embryonic aortic arch (AOAR) and the common carotid artery (CCA) during branchial arch artery development in mice. Adamts18 deficiency was found to cause abnormal development of AOAR, CCA, and the third and fourth branchial arch appendages, leading to hypoplastic carotid body, thymus, and variation of middle cerebral artery. Adamts18 was shown to affect the accumulation of extracellular matrix (ECM) components, in particular fibronectin (Fn), around AOAR and CCA. As a result of increased Fn accumulation, the Notch3 signaling pathway was activated to promote the differentiation of cranial neural crest cells (CNCCs) to vascular smooth muscle cells. These data indicate that Adamts18-mediated ECM homeostasis is crucial for the differentiation of CNCCs.
Project description:BackgroundDouble aortic arch (DAA) is an extremely rare vascular malformation, even more so when coexisting with esophageal cancer.MethodsWe report a new case of DAA with esophageal cancer recently seen at our Thoracic Tumor Clinic and review cases of DAA coexisting with esophageal cancer reported in the literature of English language from 2010 to 2020. The purposes of our literature review were to explore how to best achieve radical esophagectomy while reducing postoperative complications. The clinical manifestations, diagnostic method, surgical approach, reconstruction route, and the extent of lymphadenectomy of esophageal cancer with DAA were analyzed in detail.Results and conclusionFor such patients, 3D computed tomography is necessary for preoperative diagnosis. The surgical approach should consider factors such as the location of the tumor in the esophagus and whether the tumor is surrounded by DAA, as well as the position of the descending aorta and the requirements for the surgical field for lymphadenectomy. If esophageal reconstruction is required, the retrosternal route is preferred. We recommend that only patients with positive results of intraoperative frozen biopsy of recurrent laryngeal nerve lymph nodes should undergo three-field lymphadenectomy, which may be the best method to achieve radical esophagectomy for middle and lower esophageal cancers with DAA while minimizing postoperative complications.
Project description:Objective To report a rare case of unruptured internal carotid-posterior communicating artery (IC-PC) aneurysm splitting the oculomotor nerve treated by clipping and to review the previously published cases. Case Presentation A 42-year-old man suddenly presented with left oculomotor paresis. Three-dimensional digital subtraction angiography (3D DSA) demonstrated a left IC-PC aneurysm with a bulging part. During surgery, it was confirmed that the bulging part split the oculomotor nerve. After the fenestrated oculomotor nerve was dissected from the bulging part with a careful microsurgical technique, neck clipping was performed. After the operation, the symptoms of oculomotor nerve paresis disappeared within 2 weeks. Conclusions We must keep in mind the possibility of an anomaly of the oculomotor nerve, including fenestration, and careful observation and manipulation should be performed to preserve the nerve function during surgery, even though it is very rare.